Background: Neural tube defects (NTDs) are the most common birth defect of the central nervous system. Spina bifida (SB) is a common birth defect resulting from incomplete closure of the neural tube during the first month of pregnancy and that is associated with significant clinical complications that can affect survival and the quality of life for affected individuals. Objective: The study objective was to estimate the prevalence, types, manifestations, diagnosis, complications as well as treatment outcomes of of Spina bifida in all neonates born in Maternity and Children hospital in Arar city, Northern Saudi Arabia. Methods: This is a descriptive study involved all neonates born in Maternity and Children hospital in Arar city, Northern Saudi Arabia, KSA, during the period from 1 January to 31 December 2017. Data was collected by retrospective analysis of the recorded data and filling a pre designed questionnaire, which include questions designed to fulfill the study objectives. Results: The total number of spina bifida cases borne in maternity and children hospital in Arar city, Northern Saudi Arabia during the year 2017 was 14 cases with a prevalence of 0.51%; 8(57.1%) were females and 6(42.9%) were males. Consanguinity between parents found in 57.1% of cases, and folic acid deficiency during pregnancy reported in 78.6%. The site of spina bifida was lumbosacral in 57.1% and lumber 42.9%. The type was Spina bifida occulta 57.1%, Myelomeningocele 28.6% and Myeloschisis 14.3%. Weakness of legs, urinary incontinence 35.7%, hip dislocation, deformity of the body 21.4% and paralysis were the reported complications. Conclusion: The prevalence of spina bifida in Arar, KSAwas 5.1per 1,000 live births. Majority of the reported cases were females, Spina bifida occulta was the most common then myelomeningocele and myeloschisis. The site was lumbosacral and lumber.